Giant Cell Arteritis (Temporal Arteritis)

Giant Cell Arteritis (Temporal Arteritis)

Giant cell arteritis is a serious autoimmune disease caused by inflammation in the walls of large-to-medium arteries of the upper body. Involvement of the neck arteries and especially of the temporal branch of external carotid artery is a hallmark of the disease. This how giant cell arteritis earned its alternative name of temporal arteritis.

Inflammation in the wall narrows the blood vessel’s diameter limiting blood supply to the tissues. Permanent loss of vision in one of both eyes is the major concern in this disease.

Giant cell arteritis is rare before age 55. Mostly whites of European decent are affected.

Symptoms of Temporal Arteritis

Headache in Temporal Arteritis

Headache is normally the first symptom, which is brought to doctor’s attention. Headache in temporal arteritis is a dull and annoying pain. It is often centered in the temple but it can spread to the other side or to the whole head. Some patients have hypersensitive scalp, similar to allodynia caused by chronic migraine. The headache is rather chronic, persisting though the day but it may be intermittent as well.

The rest of the symptoms in temporal arteritis are caused by the tissue ischemia (insufficient blood supply). Tongue and jaw claudication (pain and fatigue of the jaw and tongue muscles) while chewing.

Presence of these symptoms makes the diagnosis of temporal arteritis almost certain. Unfortunately, these classical features are frequently missing and the disease hits the eye first.

Vision Loss In Temporal Arteritis

The most devastating complication of giant cell arteritis is a sudden and permanent loss of vision in one eye. The other eye may be affected later.

Ophthalmic artery blockage is responsible for the vision loss. Ophthalmic artery supplies retina, optic nerve and other structures inside the orbit. Vision loss is often permanent and it comes with no warnings.

For this reason, every person over 55 with a new type of unexplained headache has to be evaluated for giant cell arteritis; or it has to be considered, at least.

Polymyalgia Rheumatica

About 50% of patients affected by temporal arteritis have involvement of the other parts of the body leading to the diagnosis of polymyalgia rheumatica.

Polymialgia rheumatica has nonspecific symptoms of fatigue, malaise, fever, pain and stiffness in the neck and the limbs.

About 15% of untreated people with polymialgia rheumatica will eventually develop temporal arteritis.

Diagnosis of Temporal Arteritis

Diagnosis of giant cell arteritis may be challenging. Examination may reveal no pain or tenderness in the temporal artery. As much as 25% of patients have loss of vision as a first symptom of the disease. They have no systemic symptoms of autoimmune disease, such as headache, weight loss, fatigue, body ache (myalgia), or jaw claudication. Loss of vision may be bilateral.

Permanent loss of vision may be preceded by episodes of transient vision loss or double vision. For this reason, any person over 55 with a double vision and a headache has to be evaluated regarding the possibility of temporal arteritis.
Treatment of temporal arteritis is far from benign, so a definite diagnosis is very desirable.

The most reliable diagnostic test for temporal arteritis is a temporal artery biopsy (if positive). The procedure is unpleasant and may be negative in the cases with no jaw/tongue claudication, which throws us back to square one.

Blood tests for ESR and C-Reactive Protein demonstrate very high numbers (ESR is usually 80 and above). The problem with ESR is that, it is very sensitive but not specific. Later in life ESR is naturally elevated and there are lots of other reasons for the elevated ESR in this age group. On the other hand, when ESR shoots above 80 in any age group it is definitely concerning.

Any infection or inflammation in the body will make the numbers high. In 12% of cases of temporal arteritis, ESR may be normal and only CRP level is elevated. Even CRP might be normal, which adds up some extra challenge to the diagnostic process.

Elevation of CRP and ESR in response to inflammation is not simultaneous. Repeated blood work makes a perfect sense.

Ultrasound of the temporal arteries may demonstrate a “halo” sign. Doctors infrequently rely on this test, however.

MRI and even PET CT may be helpful, but they are rarely utilized for the diagnosis of temporal arteritis.

Treatment of Temporal Arteritis

Sudden loss of vision from suspected temporal arteritis requires a high dose steroid treatment (1 gram per day of methylprednisolone for 3 to 5 days) without waiting for the results of the diagnostic testing. IV steroids are later substituted for an oral formulation, see below.

Long term corticosteroids, such as Prednisone, is the mainstay of the treatment. Treatment of temporal arteritis is a double-edged sword, since the missed diagnosis often causes a permanent blindness but the incorrect one destines the patient to months and years of an unnecessary steroid treatment. Long term treatment with corticosteroids predictably leads to a wide range of side effects.

The dose of steroids is relatively high (about 1mg of Prednisone per kg). Headache due to temporal arteritis is expected to subside within a week or even sooner.

Corticosteroids are notorious for alleviating any type of headache, so the pain relief not prove the correct diagnosis. Persistence of the headache beyond one week of Prednisone treatment makes a giant cell arteritis diagnosis unlikely.

Loss of vision remains a possibility for the first month of Prednisone treatment even if the treatment has been started immediately after this disease was suspected.

Steroid treatment usually lasts from months to years (average 2 years). Giant cell arteritis is not a life-long condition. It is wise to taper steroids every a few months while watching for ESR numbers. If ESR level does not rise, steroids can be gradually discontinued.

Important! Never stop steroids without doctor’s recommendations and/or supervision because it might be life-threatening!

Any comments about this page will be greatly appreciated at doctorstrizhak@gmail.com Content copyright 2017. DOCTORSTRIZHAK.COM. All rights reserved.Disclosure: This Web Site is intended for education purpose only. The information provided on this site must not be perceived as a guide for self-diagnosis or self-treatment. Every effort is made to keep the information current, but there are absolutely no guarantees of timely updates. By Andre Strizhak